Smoyer W E, Gregory M J, Bajwa R S, Johnson K J, Bunchman T E
Department of Pediatrics, University of Michigan, Ann Arbor, USA.
Pediatr Nephrol. 1998 Nov;12(9):737-43. doi: 10.1007/s004670050536.
Use of cyclosporine (CsA) in the management of children with steroid-resistant (SRNS) and steroid-dependent (SDNS) nephrotic syndrome has become increasingly popular in recent years. Although most children receive a renal biopsy prior to initiation of CsA, the relationship between initial renal histology and the subsequent clinical response to CsA is not known. We analyzed the correlation between pre-CsA segmental and global glomerular scarring and interstitial fibrosis and the subsequent response to CsA in 23 children (5.6+/-1.0 years, Mean+/-SEM) with SDNS (n=8) and SRNS (n=15) treated with CsA for 24.2+/-3.8 months and followed for 28.0+/-4.1 months. Complete remission was obtained in 78% of patients within 67.6+/-16 days, while 18% had a partial response and 4% no response. Quantitative histological analysis revealed a trend toward partial rather than complete response with increasing segmental glomerular (P=0.13), global glomerular (P=0.05), and interstitial (P=0.08) scarring, and among patients with minimal change nephrotic syndrome versus IgM nephropathy versus focal segmental glomerulosclerosis. Among complete responders, linear regression analyses revealed no correlation between time to response and pre-CsA glomerular or interstitial scarring. We conclude that increased glomerular or interstitial scarring on a pre-CsA renal biopsy tends to correlate with a partial, rather than complete, response to CsA in childhood nephrotic syndrome.
近年来,环孢素(CsA)在激素抵抗型(SRNS)和激素依赖型(SDNS)肾病综合征患儿的治疗中应用越来越广泛。尽管大多数患儿在开始使用CsA之前会接受肾活检,但初始肾脏组织学与随后对CsA的临床反应之间的关系尚不清楚。我们分析了23例(平均年龄5.6±1.0岁,均值±标准误)SDNS(n = 8)和SRNS(n = 15)患儿在接受CsA治疗24.2±3.8个月并随访28.0±4.1个月时,CsA治疗前节段性和全球性肾小球瘢痕形成及间质纤维化与随后对CsA反应之间的相关性。78%的患者在67.6±16天内完全缓解,18%部分缓解,4%无反应。定量组织学分析显示,随着节段性肾小球(P = 0.13)、全球性肾小球(P = 0.05)和间质(P = 0.08)瘢痕形成增加,有部分而非完全缓解的趋势,且在微小病变肾病、IgM肾病和局灶节段性肾小球硬化症患者之间也存在这种趋势。在完全缓解者中,线性回归分析显示缓解时间与CsA治疗前肾小球或间质瘢痕形成之间无相关性。我们得出结论,CsA治疗前肾活检中肾小球或间质瘢痕形成增加往往与儿童肾病综合征对CsA的部分而非完全反应相关。